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Drug reaction with eosinophilia and systemic symptoms (DRESS) is a severe, adverse drug reaction that is notoriously complex in both its presentation and treatment. Although early diagnosis and cessation of the causative agent are universally accepted as the initial interventions for DRESS, the subsequent management lacks a standardized approach. Historically, systemic steroids have been used as first-line treatment, but there is debate about the optimal dosing and route of administration, and evidence persists on the long-term complications associated with steroid use. Novel treatment approaches with targeted therapy, cyclosporine, intravenous immunoglobulin, and plasmapheresis have been gaining interest as alternative mono- and adjuvant therapies, but their use has yet to be supported by clinical trials. This narrative review provides a summary of the current knowledge of DRESS, with a focus on clinical management. The various mono- and adjuvant therapy options are discussed, with literature-supported suggestions for their optimal use in clinical practice. The risks for relapses, viral reactivation, and long-term complications are also considered. The PubMed and Medline databases were searched for articles on DRESS, published between January 1, 2008, and May 1, 2023. 334 articles met the inclusion criteria. Based on the literature, a DRESS management tool with step-by-step guidance is provided. Further suggestions for management are woven throughout this review, giving clinicians a toolbelt of resources with which to approach diagnosis, treatment, and follow-up. [ABSTRACT FROM AUTHOR]

This article summarized clinical data and nursing management of a patient with drug-induced hypersensitivity syndrome characterized by toxic epidermal necrolysis and dermatitis combined with multiple organ failure treated with continuous renal replacement therapy (CRRT). Given the complex illness condition of the patient, a comprehensive evaluation on nursing risks was carried out before CRRT. In addition to enhance the nursing cooperation in CRRT, nurses had improved the nursing interventions on symptoms of multiple organ failure, catheter maintanence, skin care, diet guildance and health education during the hospitalizaiton. After effective treatment and meticulous care, the patient's skin condition and psychological state were improved. (本文总结1例以中毒性表皮坏死松解症为表现特征的药物超敏反应综合征样皮炎合并多脏器衰竭患者行连续性肾脏替代治疗(CRRT)的护理经验。患者病情复杂, 入院后积极完善评估和检查, 采取CRRT治疗, 同时加强脏器衰竭护理、CRRT护理、管路护理、皮肤护理, 做好饮食指导和健康宣教, 积极预防并发症, 患者的皮损明显减轻, 生命体征相对恢复稳定。)

OBJECTIVES: To describe and discuss a rare adverse reaction to drugs diagnosed in an elderly female patient after using levofloxacin and metronidazole: the DRESS syndrome (Drug Rash with Eosinophilia and Systemic Symptoms). CASE DESCRIPTION: A 77-year-old elderly woman was diagnosed with pneumonia. After undergoing treatment with metronidazole and levofloxacin, she developed pruritic skin lesions, eosinophilia, and fever. INVESTIGATIONS: We established a suspected diagnosis of levofloxacin-induced DRESS syndrome, and therefore we switched the antibiotics and then administered corticotherapy. The patient exhibited rapid and progressive improvement without damage to other organs. DIFFERENTIAL DIAGNOSIS: Conditions involving eosinophilia, drug hypersensitivity, and/or skin rash. COMMENTS: This syndrome is characterized by skin eruption, systemic symptoms, and eosinophilia. Although the patient did not meet all clinical criteria in the literature, the lack of consensus among authors means that a DRESS syndrome diagnosis could not be ruled out. The condition is rare, but clinicians should be alert to this diagnosis in aged individuals, given its severity and high risk of mortality.

Introduction: Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a potentially life-threatening, drug-induced adverse reaction characterized by skin eruptions, lymphadenopathy, fever, and a broad range of other bodily manifestations. The spectrum of histopathologic and clinical presentations is wide; therefore, DRESS syndrome can mimic other diseases. Case Presentation: We present a case of a 4-year-old male patient who started chemotherapy with vincristine, cytarabine, and etoposide. The first clinical signs were fever, hemodynamic in-stability, and maculopapular erythema. Biopsies of skin lesions were taken, and hyperkeratosis, focal parakeratosis, acanthosis with slight spongiosis, and intraepithelial dyskeratotic cells were observed. There was a perivascular lymphoid infiltrate with abundant eosinophils in the dermis, and eosinophil permeations to the acrosyringium and epithelium were found. Conclusion: DRESS syndrome is a drug-induced reaction that shares histopathological findings in skin biopsies with those seen in graft-versus-host disease. Although the histological findings are non-pathognomonic, they were characteristic enough to be of importance in the differential diagnosis.

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Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) is a complex and potentially fatal hypersensitivity condition. We present a unique case report and literature review focusing on DRESS syndrome-associated myocarditis resulting from reactivated viral infections in a 21-year-old female. 3 weeks after 5-day oral co-trimoxazole consumption due to acne, she developed symptoms consistent with DRESS syndrome, including a generalized maculopapular rash. Despite prednisolone treatment, the patient developed fatal fulminant myocarditis linked to HHV-6 and CMV reactivation. The patient's death highlights the importance of early recognition and careful management of DRESS syndrome, especially considering the potential viral reactivation that can lead to severe complications. Postmortem investigations revealed that viral reactivation caused myocarditis. Careful consideration must be given to corticosteroid usage in DRESS treatment, as inappropriate prescribing may promote viral reactivation and subsequent complications. While high-dose corticosteroids initiated within the first week effectively suppress HHV-6 reactivation. Conversely, low-dose or late-start high-dose corticosteroids prove ineffective in preventing HHV-6 viremia. Late- onset or low- dose corticosteroids may lead to fatal complications following the primary viral reactivation. [ABSTRACT FROM AUTHOR]

Abstract Background Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) Syndrome is a severe adverse drug reaction marked by delayed hypersensitivity reactions causing skin and systemic complications. DRESS diagnosis is challenging due to the variety of clinical presentations and symptom overlap with other conditions. The perioperative period in these patients requires precise pharmacological strategies to prevent complications associated with this syndrome. The treatment of DRESS induced by unfractionated heparin during cardiopulmonary bypass (CPB) surgery presents some challenges that must be considered when selecting an anticoagulant to avoid side effects. In this case, bivalirudin, a direct thrombin inhibitor, is indicated as an alternative to heparin in patients undergoing CPB. However, in contrast to heparin/protamine, there is no direct reversal agent for bivalirudin. Case presentation We report the case of an 11-year-old male diagnosed with native aortic valve endocarditis and thrombosis in his left lower extremity. During valvular replacement surgery, systemic unfractionated heparin was administered. Postoperatively, the patient developed fever, eosinophilia and pruritic rash. Warm shock and elevated alanine transaminase (ALT) and aspartate transaminase (AST) levels followed, leading to the diagnosis of DRESS syndrome. Treatment with methylprednisolone resulted in complete resolution of symptoms. Seven years later, the patient was readmitted due to insufficient anticoagulation and a thrombus in the prosthetic aortic valve, presenting a recurrent DRESS episode due to the administration of unfractionated heparin, which was later replaced with low-molecular-weight heparin during hospitalization. Treatment with corticosteroids and antihistamines was initiated, resulting in the resolution of this episode. Ultimately, the patient required the Ross procedure. During this intervention the anticoagulation strategy was modified, unfractionated heparin was replaced with bivalirudin during the procedure and fondaparinux was administered during the postoperative period. This resulted in stable transaminases levels and no eosinophilia. Conclusion The severity of DRESS Syndrome underscores the importance of early recognition, heightened monitoring, and a comprehensive approach tailored to each patient’s needs. This particular case highlights the significance of this approach and may have a substantial clinical impact since it provides alternatives to heparin, such as bivalirudin and fondaparinux, in the anticoagulation strategy of CPB for patients who have a hypersensibility reaction to this medication; thus, enhancing clinical outcomes by minimizing risks linked to adverse drug reactions.

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a rare, life-threatening adverse reaction caused by certain medications. Clinical findings usually include rash, fever, lymphadenopathy, and eosinophilia, and in some cases, they may affect major organs. This reaction caused by antituberculosis (TB) medication poses a public health risk due to treatment discontinuation, adherence, or success in cure. We present a 23-year-old female patient who developed DRESS syndrome as a result of group A anti-TB drugs (ATDs), an exceedingly rare occurrence. The patient’s medication was successfully retrieved using a re-desensitization protocol.

Background: Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) Syndrome is a severe adverse drug reaction marked by delayed hypersensitivity reactions causing skin and systemic complications. DRESS diagnosis is challenging due to the variety of clinical presentations and symptom overlap with other conditions. The perioperative period in these patients requires precise pharmacological strategies to prevent complications associated with this syndrome. The treatment of DRESS induced by unfractionated heparin during cardiopulmonary bypass (CPB) surgery presents some challenges that must be considered when selecting an anticoagulant to avoid side effects. In this case, bivalirudin, a direct thrombin inhibitor, is indicated as an alternative to heparin in patients undergoing CPB. However, in contrast to heparin/protamine, there is no direct reversal agent for bivalirudin. Case presentation: We report the case of an 11-year-old male diagnosed with native aortic valve endocarditis and thrombosis in his left lower extremity. During valvular replacement surgery, systemic unfractionated heparin was administered. Postoperatively, the patient developed fever, eosinophilia and pruritic rash. Warm shock and elevated alanine transaminase (ALT) and aspartate transaminase (AST) levels followed, leading to the diagnosis of DRESS syndrome. Treatment with methylprednisolone resulted in complete resolution of symptoms. Seven years later, the patient was readmitted due to insufficient anticoagulation and a thrombus in the prosthetic aortic valve, presenting a recurrent DRESS episode due to the administration of unfractionated heparin, which was later replaced with low-molecular-weight heparin during hospitalization. Treatment with corticosteroids and antihistamines was initiated, resulting in the resolution of this episode. Ultimately, the patient required the Ross procedure. During this intervention the anticoagulation strategy was modified, unfractionated heparin was replaced with bivalirudin during the procedure and fondaparinux was administered during the postoperative period. This resulted in stable transaminases levels and no eosinophilia. Conclusion: The severity of DRESS Syndrome underscores the importance of early recognition, heightened monitoring, and a comprehensive approach tailored to each patient's needs. This particular case highlights the significance of this approach and may have a substantial clinical impact since it provides alternatives to heparin, such as bivalirudin and fondaparinux, in the anticoagulation strategy of CPB for patients who have a hypersensibility reaction to this medication; thus, enhancing clinical outcomes by minimizing risks linked to adverse drug reactions. [ABSTRACT FROM AUTHOR]

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a rare, life-threatening adverse reaction caused by certain medications. Clinical findings usually include rash, fever, lymphadenopathy, and eosinophilia, and in some cases, they may affect major organs. This reaction caused by antituberculosis (TB) medication poses a public health risk due to treatment discontinuation, adherence, or success in cure. We present a 23-year-old female patient who developed DRESS syndrome as a result of group A anti-TB drugs (ATDs), an exceedingly rare occurrence. The patient's medication was successfully retrieved using a re-desensitization protocol. [ABSTRACT FROM AUTHOR]

Purpose of review: This review describes risk factors for severe skin reactions to antiseizure medications (ASMs), the usage of updated tests to predict those with increased risk of a severe cutaneous reaction, and guides how to choose specific ASMs and dosing to lower the risk for these reactions. Information is given regarding specific mild versus severe reactions, initial diagnostic evaluation, and treatment. A table listing the risk of mild and severe cutaneous reaction risks as well as the management of potential seizures that may occur while stopping the culprit ASM are provided. Recent findings: Five new ASMs have joined the total of 26 FDA-approved ASMs since 2018. Cenobamate had three patients develop a drug reaction with eosinophilia and systemic symptoms. A lower starting dosing and slower titration have resulted in no further published cases. Based on limited data, rash risk is low for fenfluramine, ganaxalone, and stiripentol. It is low-moderate for Epidiolex. Molecular tests can predict severe reactions. Summary: Skin reactions are a relatively common side effect of ASMs with aromatic ASMs having the greatest risk. Identifying and informing high-risk patients when to seek medical attention, stopping the culprit ASM when a severe reaction looks possible, and providing appropriate medical triage can reduce morbidity and mortality from severe skin and systemic reactions. [ABSTRACT FROM AUTHOR]

BACKGROUND: Drug Reaction Syndrome with Eosinophilia and Systemic Symptoms (DRESS) is an adverse drug reaction, being life-threatening in 10% of cases. CLINICAL CASE: 15-year-old male patient attended the pediatric emergency department with a clinical history of morbilliform and pruritic exanthema on the face and neck of 9 days of evolution, extending to the thorax and extremities, accompanied by fever of 38.0°C of 1 day of evolution. The patient had a history of atopy and acne vulgaris, treated with minocycline. On admission the hemogram showed leukocytes 16,900 cells/ml, eosinophils 18.2%, neutrophils 6.2%. Blood chemistry revealed: creatinine 1.92 mg/dl, TSGO 45 U/L, TSGP 287 U/L, CRP: 62.8 mg/L and ESR 21 mm/h. It was decided to admit him to the pediatric intensive care unit, since he complies with 7 RegiSCAR points. CONCLUSION: DRESS syndrome is a severe and potentially fatal reaction; its diagnosis is a challenge due to its heterogeneity and clinical variability. It should be taken into consideration after a detailed interrogation that yields the use of suspicious drugs prior to the onset of symptoms, even though its incidence has not been fully described. [ABSTRACT FROM AUTHOR]

Background and Aims: Adverse cutaneous drug reactions (ACDRs) are common and potentially life‐threatening, while also hindering patient compliance to medications. Given the regional differences in patterns and prevalence of ACDRs, it is important to study the epidemiology, as well as the clinical and outcome patterns of patients with ACDRs in Iran. Methods: This cross‐sectional study on ACDRs was conducted among hospitalized patients in a referral university hospital in the city of Isfahan, Iran. The patients' demographics, clinical information, and outcomes, including age, gender, past medical history, medication history, drug reaction with eosinophilia and systemic symptoms (DRESS) diagnosis, Steven‐Johnson Syndrome (SJS) diagnosis, toxic epidermal necrosis (TEN) diagnosis, treatment regimen (steroids or intravenous immunoglobulin [IVIG]) and outcome information, including intensive care requirements, severe medical complications, or death, were obtained from medical records. Results: A total of 195 patients with a mean age of 40 years and consisting of 61% females were included. Carbamazepine, lamotrigine, sodium valproate, and phenytoin are the most commonly reported medications. Rate of complications was 45% with DRESS, SJS, and TEN diagnosed in 26%, 47%, and 19%, respectively. Treatment was carried out with steroids and IVIG in 81% and 19%, respectively. Among patients, 15% required intensive care and 5% died. Diagnosis of TEN, older age, and baseline heart disease were predictors of mortality. Patients with SJS were younger and more likely to be males, and they were more likely to have eye complications. On the other hand, patients with the diagnosis of TEN were more likely to receive IVIG and intensive care, and had a higher mortality rate. Conclusion: Our study provides insight into the demographics and clinical patterns of Iranian patients with ACDRs. This will help in predicting rates of complications, treatments, and outcomes in patients and therefore make proper management decisions. [ABSTRACT FROM AUTHOR]

Drug reaction with eosinophilia and systemic symptoms (DRESS)/drug-induced hypersensitivity syndrome (DIHS) is a life-threatening, multi-organ adverse drug reaction with a mortality rate of approximately 10 %–20 %. The most common culprit drugs are anticonvulsants, some antibiotics such as dapsone and minocycline, salazosulfapyridine, allopurinol and some antiretroviral molecules such as abacavir and nevirapine. Only one case of DRESS induced by sildenafil has been reported in the literature. Here we report a new case.

Introduction: The paediatric population is prone to developing cutaneous adverse drug reactions. However, the incidence of Severe Cutaneous Adverse Drug Reactions (SCAR) is rare in this age group, with few studies describing such reactions in detail. Aim: To describe the clinico-epidemiological factors, drug profile, laboratory parameters, and treatment outcomes of SCAR in children admitted to a tertiary care centre in South India. Materials and Methods: A retrospective cohort study was carried out over a 10-year period, including paediatric patients (0-18 years) admitted to Dermatology, Medicine, and Paediatric wards in the tertiary care centre. Demographic details, suspected drugs, comorbidities, personal and family history of drug reactions, physical examination, laboratory parameters, treatment received along with its duration, and the state of morbidity and mortality were recorded. SPSS version 18.0 was used for analysis. Descriptive statistics were used to summarise the demographics and clinical characteristics of the patients. Results: Among all the patients admitted with SCAR, 27 (15%) belonged to the paediatric age group. The median age was 15 years, and the female-to-male ratio was 1.25. Nineteen (70.3%) were diagnosed with Stevens-Johnson Syndrome (SJS)/Toxic Epidermal Necrolysis (TEN), and eight (29.6%) were diagnosed with Drug Reaction with Eosinophilia and Systemic Symptom (DRESS). There were no cases of Acute Generalised Exanthematous Pustulosis (AGEP). The most common class of drugs implicated was antiepileptics (62.8%). Two patients (7%) had a family history of drug reactions. All patients had mucosal involvement. The majority of the children responded to intravenous steroids, and two required additional intravenous immunoglobulin injections for clinical improvement. All cases were cured with no mortality or long-term sequelae. Conclusion: The incidence of SCAR in the paediatric age group is significant. Anticonvulsants, particularly phenytoin, carbamazepine, and lamotrigine, need to be used with caution in this age group. Prompt diagnosis and treatment with systemic steroids can reduce mortality, morbidity, and long-term sequelae.

OBJECTIVES: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a severe, delayed hypersensitivity reaction (DHR). We observed DRESS to inhibitors of interleukin 1 (IL-1) or IL-6 in a small group of patients with Stills disease with atypical lung disease. We sought to characterise features of patients with Stills disease with DRESS compared with drug-tolerant Stills controls. We analysed human leucocyte antigen (HLA) alleles for association to inhibitor-related DHR, including in a small Kawasaki disease (KD) cohort. METHODS: In a case/control study, we collected a multicentre series of patients with Stills disease with features of inhibitor-related DRESS (n=66) and drug-tolerant Stills controls (n=65). We retrospectively analysed clinical data from all Stills subjects and typed 94/131 for HLA. European Stills-DRESS cases were ancestry matched to International Childhood Arthritis Genetics Consortium paediatric Stills cases (n=550) and compared for HLA allele frequencies. HLA association also was analysed using Stills-DRESS cases (n=64) compared with drug-tolerant Stills controls (n=30). KD subjects (n=19) were similarly studied. RESULTS: Stills-DRESS features included eosinophilia (89%), AST-ALT elevation (75%) and non-evanescent rash (95%; 88% involving face). Macrophage activation syndrome during treatment was frequent in Stills-DRESS (64%) versus drug-tolerant Stills (3%; p=1.2×10-14). We found striking enrichment for HLA-DRB1*15 haplotypes in Stills-DRESS cases versus INCHARGE Stills controls (p=7.5×10-13) and versus self-identified, ancestry-matched Stills controls (p=6.3×10-10). In the KD cohort, DRB1*15:01 was present only in those with suspected anakinra reactions. CONCLUSIONS: DRESS-type reactions occur among patients treated with IL-1/IL-6 inhibitors and strongly associate with common HLA-DRB1*15 haplotypes. Consideration of preprescription HLA typing and vigilance for serious reactions to these drugs are warranted.

Abstract Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) syndrome is a rare but severe and sometimes fatal adverse drug reaction that is known to occur with a number of antiepileptic drugs. It often follows a prolonged clinical course, which can worsen even after discontinuing the causative drug and administering steroid treatment. Failure to promptly identify the delayed involvement of vital organs, such as the heart and liver, may result in irreversible organ failure and death. We report a case of a presumed sudden death of a young woman who had a documented history of a protracted intermittent hypersensitivity reaction to lamotrigine. Postmortem examination revealed the presence of eosinophilic myocarditis and submassive hepatic necrosis diagnostic of fatal DRESS syndrome that progressed despite early discontinuation of the medication and improvement of dermatologic and hematologic symptoms following steroid therapy.

Abstract Background and Aims Adverse cutaneous drug reactions (ACDRs) are common and potentially life‐threatening, while also hindering patient compliance to medications. Given the regional differences in patterns and prevalence of ACDRs, it is important to study the epidemiology, as well as the clinical and outcome patterns of patients with ACDRs in Iran. Methods This cross‐sectional study on ACDRs was conducted among hospitalized patients in a referral university hospital in the city of Isfahan, Iran. The patients' demographics, clinical information, and outcomes, including age, gender, past medical history, medication history, drug reaction with eosinophilia and systemic symptoms (DRESS) diagnosis, Steven‐Johnson Syndrome (SJS) diagnosis, toxic epidermal necrosis (TEN) diagnosis, treatment regimen (steroids or intravenous immunoglobulin [IVIG]) and outcome information, including intensive care requirements, severe medical complications, or death, were obtained from medical records. Results A total of 195 patients with a mean age of 40 years and consisting of 61% females were included. Carbamazepine, lamotrigine, sodium valproate, and phenytoin are the most commonly reported medications. Rate of complications was 45% with DRESS, SJS, and TEN diagnosed in 26%, 47%, and 19%, respectively. Treatment was carried out with steroids and IVIG in 81% and 19%, respectively. Among patients, 15% required intensive care and 5% died. Diagnosis of TEN, older age, and baseline heart disease were predictors of mortality. Patients with SJS were younger and more likely to be males, and they were more likely to have eye complications. On the other hand, patients with the diagnosis of TEN were more likely to receive IVIG and intensive care, and had a higher mortality rate. Conclusion Our study provides insight into the demographics and clinical patterns of Iranian patients with ACDRs. This will help in predicting rates of complications, treatments, and outcomes in patients and therefore make proper management decisions.

Background: Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a rare hypersensitivity reaction involving the skin and various visceral organs; the kidneys are the second most affected organ. Many drugs are reported to be associated with DRESS, particularly antiepileptic agents and allopurinol. Certain human leukocyte antigen (HLA) haplotypes, in combination with a particular drug, can further contribute to an increased risk of DRESS. Symptoms often develop 2 to 8 weeks after drug initiation. If diagnosis is delayed, DRESS can be a life-threatening condition. Case Reports: We present cases of 2 patients. The first patient was an 86-year-old Polish woman who developed acute kidney injury and skin lesions with accompanying leucocytosis and eosinophilia during long-term antibiotic therapy with piperacillin/tazobactam and ciprofloxacin. The second patient was a 37-year-old Asian woman with predialysis chronic renal disease stage V in the course of IgA nephropathy. Two weeks after starting allopurinol in a standard dose, she presented with maculopapular rash, facial edema, fever, liver injury, and eosinophilia. Renal function started to deteriorate, but she did not require dialysis. In both cases, the discontinuation of the above-mentioned drugs and the introduction of steroid therapy and intravenous immunoglobulins allowed for clinical improvement and recovery. In the second case, the extended 4-locus HLA typing was performed retrospectively, and allele HLA-B*5801 was found. Conclusions: Due to the rare occurrence and heterogeneous manifestation of DRESS, its diagnosis can pose many difficulties. In-depth analysis of symptoms, medicines taken, and laboratory findings enable the implementation of appropriate treatment and recovery. [ABSTRACT FROM AUTHOR]

Introduction: The paediatric population is prone to developing cutaneous adverse drug reactions. However, the incidence of Severe Cutaneous Adverse Drug Reactions (SCAR) is rare in this age group, with few studies describing such reactions in detail. Aim: To describe the clinico-epidemiological factors, drug profile, laboratory parameters, and treatment outcomes of SCAR in children admitted to a tertiary care centre in South India. Materials and Methods: A retrospective cohort study was carried out over a 10-year period, including paediatric patients (0-18 years) admitted to Dermatology, Medicine, and Paediatric wards in the tertiary care centre. Demographic details, suspected drugs, comorbidities, personal and family history of drug reactions, physical examination, laboratory parameters, treatment received along with its duration, and the state of morbidity and mortality were recorded. SPSS version 18.0 was used for analysis. Descriptive statistics were used to summarise the demographics and clinical characteristics of the patients. Results: Among all the patients admitted with SCAR, 27 (15%) belonged to the paediatric age group. The median age was 15 years, and the female-to-male ratio was 1.25. Nineteen (70.3%) were diagnosed with Stevens-Johnson Syndrome (SJS)/Toxic Epidermal Necrolysis (TEN), and eight (29.6%) were diagnosed with Drug Reaction with Eosinophilia and Systemic Symptom (DRESS). There were no cases of Acute Generalised Exanthematous Pustulosis (AGEP). The most common class of drugs implicated was antiepileptics (62.8%). Two patients (7%) had a family history of drug reactions. All patients had mucosal involvement. The majority of the children responded to intravenous steroids, and two required additional intravenous immunoglobulin injections for clinical improvement. All cases were cured with no mortality or long-term sequelae. Conclusion: The incidence of SCAR in the paediatric age group is significant. Anticonvulsants, particularly phenytoin, carbamazepine, and lamotrigine, need to be used with caution in this age group. Prompt diagnosis and treatment with systemic steroids can reduce mortality, morbidity, and long-term sequelae. [ABSTRACT FROM AUTHOR]

Purpose of Review: As a sulfone antibacterial agent, dapsone has been widely used to treat leprosy. Moreover, dapsone is also used in many immune diseases such as herpetic dermatitis because of its anti-inflammatory and immunomodulatory effects. However, dapsone can cause several adverse effects, the most serious being dapsone hypersensitivity syndrome. Dapsone hypersensitivity syndrome is characterized by a triad of eruptions, fever, and organ involvement, which limits the application of dapsone to some extent. Recent Findings: In this article, we review current research about the interaction model between HLA-B*13:01, dapsone, and specific TCR in dapsone-induced drug hypersensitivity. In addition to the proposed mechanisms, we also discussed clinical features, treatment progress, prevalence, and prevention of dapsone hypersensitivity syndrome. Summary: These studies reveal the pathogenesis, clinical features, and prevalence from the perspectives of genetic susceptibility and innate and adaptive immunity in dapsone hypersensitivity syndrome, thereby guiding clinicians on how to diagnose, prevent, and treat dapsone hypersensitivity syndrome. [ABSTRACT FROM AUTHOR]

Stevens-Johnson syndrome is an infrequent condition affecting the skin and mucous membranes, it involves cutaneous detachment with high mortality without adequate treatment. We present the case of a 40-year-old male with a history of epilepsy treated with valproic acid and lamotrigine, previously diagnosed with dengue. Evaluation showed erythematous blisters on skin and mucosa with bleeding and desquamation, covering 10% of the body surface. The patient progressed favorably with the medical care received. Stevens-Johnson syndrome should be studied in association with arboviral diseases. [ABSTRACT FROM AUTHOR]

Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) syndrome is a rare but severe and sometimes fatal adverse drug reaction that is known to occur with a number of antiepileptic drugs. It often follows a prolonged clinical course, which can worsen even after discontinuing the causative drug and administering steroid treatment. Failure to promptly identify the delayed involvement of vital organs, such as the heart and liver, may result in irreversible organ failure and death. We report a case of a presumed sudden death of a young woman who had a documented history of a protracted intermittent hypersensitivity reaction to lamotrigine. Postmortem examination revealed the presence of eosinophilic myocarditis and submassive hepatic necrosis diagnostic of fatal DRESS syndrome that progressed despite early discontinuation of the medication and improvement of dermatologic and hematologic symptoms following steroid therapy. [ABSTRACT FROM AUTHOR]

Anagen effluvium (AE) is a hair growth disorder that occurs due to a disturbance in the hair follicle cycling. The most common cause of AE is alopecia areata (AA). Autoimmune diseases like AA may develop after drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome and are usually long-standing conditions. We report the case of a 17-year-old woman who developed a severe and abrupt AE after DRESS syndrome with a full and rapid recovery after two months of topical minoxidil.

Unlike other adverse drug reactions, visceral organ involvement is a prominent feature of drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome and correlates with mortality. The aim of this study was to systematically review cases published in PubMed-indexed, peer-reviewed journals in which patients had renal injury during the episode of DRESS syndrome (DS). We found 71 cases, of which 67 were adults and 56% were males. Female sex was associated with higher mortality. Chronic kidney disease (CKD) was present in 14% of patients who developed acute kidney injury (AKI) during DS. In 21% of cases, the kidneys were the only visceral organ involved, while 54% of patients had both liver and kidney involvement. Eosinophilia was absent in 24% of patients. The most common classes of medication associated with renal injury in DS were antibiotics in 34%, xanthine oxidase inhibitors in 15%, and anticonvulsants in 11%. Among antibiotics, vancomycin was the most common culprit in 68% of patients. AKI was the most common renal manifestation reported in 96% of cases, while isolated proteinuria or hematuria was present in only 4% of cases. In cases with AKI, 88% had isolated increase in creatinine and decrease in glomerular filtration (GFR), 27% had AKI concomitantly with proteinuria, 18% had oliguria, and 13% had concomitant AKI with hematuria. Anuria was the rarest manifestation, occurring in only 4% of patients with DS. Temporary renal replacement therapy was needed in 30% of cases, and all but one patient fully recovered renal function. Mortality of DS in this cohort was 13%, which is higher than previously reported. Medication class, latency period, or pre-existing CKD were not found to be associated with higher mortality. More research, particularly prospective studies, is needed to better recognize the risks associated with renal injury in patients with DS. The development of disease-specific biomarkers would also be useful so DS with renal involvement can be easier distinguished from other eosinophilic diseases that might affect the kidney. [ABSTRACT FROM AUTHOR]

Drug reaction with eosinophilia and systemic symptoms (DRESS) is a severe cutaneous drug adverse reaction characterized by various cutaneous and systemic manifestations. However, reports on the various patterns of alopecia after DRESS are lacking. Thus, we aimed to describe cases of alopecia after DRESS and review the literature. This multicentric retrospective study reviewed the records of 182 patients diagnosed with DRESS from 2009 to 2021; of these, 10 who had alopecia after DRESS were included. Patients were diagnosed with permanent alopecia (n = 4), telogen effluvium (n = 5), and alopecia areata (n = 1), and were treated with topical minoxidil or alfatradiol (6; 60%), topical corticosteroids (3; 30%), dietary supplements (6; 60%), systemic corticosteroids (1; 10%), and intralesional corticosteroid injection (2; 20%). Although patients with permanent alopecia did not show hair regrowth after 6 months, those with telogen effluvium and alopecia areata experienced marked clinical improvement within 6 months. Various types of alopecia can persist over an extended period, even after the resolution of an acute episode of DRESS. [ABSTRACT FROM AUTHOR]

DRESS (drug reaction syndrome with eosinophilia and systemic symptoms) is a rare drug-induced multisystemic hypersensitivity response that can induce a severe cutaneous adverse reaction that is difficult to diagnose and treat. It frequently manifests as an extensive skin rash, systemic symptoms, lymphadenopathy, visceral organ involvement, and hematological alterations, mainly leukocytosis, eosinophilia, and sometimes atypical lymphocytosis that manifest 2 to 8 weeks after continuous administration of the responsible drug. The most prevalent drugs related with this syndrome are phenytoin, carbamazepine, allopurinol, and abacavir. Some specific human leukocyte antigen (HLA) alleles have been identified that are associated with hypersensitivity to these drugs. The pathophysiology of DRESS syndrome is not yet fully understood; the main hypothesis is a T-cell mediated hypersensitivity response when interacting with the major histocompatibility complex receptor in individuals with genetic susceptibility factors. The criteria of the European Registry of Severe Cutaneous Adverse Reactions to Drugs (RegiSCAR) are the most commonly used for the diagnosis of DRESS syndrome. Drug-induced hypersensitivity syndrome (DiHS), Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), and acute generalized exanthematous pustulosis (AGEP) should be considered for any rash that appears following the administration of any drug. Therapy of DRESS includes the elimination of the causative agent as soon as possible, as well as systemic corticosteroids which are the cornerstones of treatment. Steroid-sparing agents such as cyclosporine, intravenous immunoglobulins (IVIGs), and other immunosuppressive agents have been used successfully to contribute to treat- ment. [ABSTRACT FROM AUTHOR]

Background/Objectives: Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a potentially life‐threatening hypersensitive disorder. Cyclosporine has been indicated for adverse cutaneous drug eruptions. However, studies evaluating its clinical effectiveness in DRESS syndrome have been rare. This study aimed to evaluate the clinical efficacy of cyclosporine in DRESS syndrome compared to that of systemic corticosteroids. Methods: In the cyclosporine group, oral cyclosporine was administered twice a day for a total of 2–3 mg/kg/day for 1 week, and subsequently reduced to 1–1.5 mg/kg/day for extended treatment. In the corticosteroid group, intravenous or oral methylprednisolone was administered at 1–1.5 mg/kg/day for 1 week, with variable tapering plans. Laboratory changes before and after treatment, hospitalized days, treatment periods, and time to normalization from clinical manifestations in each group were statistically evaluated. Adverse effects of these regimens were observed during the entire treatment period. Results: Eighty patients were enrolled in this retrospective study. The cyclosporine and corticosteroid group had 27 and 53 patients, respectively. Total leucocyte and eosinophil counts, liver enzymes, and C‐reactive proteins were significantly decreased after treatment in both groups. There were no statistically significant differences observed in hospitalized days, treatment period, and time to normalization from clinical manifestations between the two groups. The corticosteroid group experienced relatively more adverse effects than the cyclosporine group. Conclusions: Cyclosporine was discovered to be clinically effective in DRESS syndrome and this study suggests that cyclosporine could be a feasible primary therapeutic option for DRESS syndrome. [ABSTRACT FROM AUTHOR]

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome in children is a rare adverse drug reaction with skin rash, fever, hematologic abnormalities, and multiorgan involvement. The diagnosis is difficult because of its various clinical presentations, nonetheless is extremely important due to the mortality rate. We describe a 14‐year‐old boy who developed hypersensitivity to either teicoplanin or meropenem therapy. After failing to improve with corticosteroids, he was successfully treated with therapeutic plasma exchange (TPE). TPE should be considered in the treatment with corticosteroid‐resistant DRESS syndrome. [ABSTRACT FROM AUTHOR]

BACKGROUND: DRESS syndrome (drug reaction with eosinophilia and systemic symptoms) is associated with exposure to certain drugs. It is potentially fatal (20%), with significant dermatological manifestations and visceral involvement. The main treatment is the immediate withdrawal of the suspected drug, as well as the use of systemic corticosteroids. CLINICAL CASE: A 61-year-old female patient, who, after two weeks of ampicillin consumption, presented a generalized morbilliform skin rash, non-involving soles and palms, and also itchy and painful symmetrical erythematous plaques with lamellar scaling on the face and edema of lips and oral mucosa. Laboratory tests showed leukocytosis with eosinophilia and lymphocytosis as well as impaired kidney and liver function. The histopathological study described hyperkeratosis, spongiosis, and inflammatory infiltrates in the superficial dermis. CONCLUSIONS: DRESS syndrome is a rare and potentially fatal drug reaction, which should be suspected in the presence of a morbilliform rash, fever, hypereosinophilia, as well as visceral involvement, to establish a timely diagnosis and treatment to avoid complications such as multi-organ failure, as well as mortality. [ABSTRACT FROM AUTHOR]

DRESS (drug reaction with eosinophilia and systemic symptoms) syndrome, also known as DIHS (drug-induced hypersensitivity syndrome), is an adverse and severe drug reaction. It has an estimated incidence of 1 case per 10,000 exposures to anticonvulsants, such as carbamazepine. The pathogenesis is considered type IVb hypersensitivity reaction triggered by the exposure of the related drug. The clinical presentation is characterized by fever, widespread skin lesions, internal organ compromise, a prolonged latent period and clinical course, and by a possible sequential reactivation of various human herpesvirus (HHV). The diagnosis of DRESS and DIHS is based on criteria established by the European Registry of Severe Cutaneous Adverse Reactions (RegiSCAR) and by the Japanese Research Committee on Severe Cutaneous Adverse Reaction (J-SCAR), respectively. Generally, the histopathological characteristics are not specific, as they are described as spongiotic dermatitis and drug reaction that commonly has eosinophils and apoptotic keratinocytes. Initial management requires the identification and prompt withdrawal of the related drug, besides support measures; nevertheless, corticosteroids are the mainstay treatment. The estimated mortality is 10%, primarily due to hepatic dysfunction. [ABSTRACT FROM AUTHOR]

Background: After introducing IL-1/IL-6 inhibitors, some patients with Still and Still-like disease developed unusual, often fatal, pulmonary disease. This complication was associated with scoring as DReSS (drug reaction with eosinophilia and systemic symptoms) implicating these inhibitors, although DReSS can be difficult to recognize in the setting of systemic inflammatory disease., Objective: To facilitate recognition of IL-1/IL-6 inhibitor-DReSS in systemic inflammatory illnesses (Still/Still-like) by looking at timing and reaction-associated features. We evaluated outcomes of stopping or not stopping IL-1/IL-6 inhibitors after DReSS reaction began., Methods: In an international study collaborating primarily with pediatric specialists, we characterized features of 89 drug-reaction cases versus 773 drug-exposed controls and compared outcomes of 52 cases stopping IL-1/IL-6 inhibitors with 37 cases not stopping these drugs., Results: Before the reaction began, drug-reaction cases and controls were clinically comparable, except for younger disease-onset age for reaction cases with preexisting cardiothoracic comorbidities. After the reaction began, increased rates of pulmonary complications and macrophage activation syndrome differentiated drug-reaction cases from drug-tolerant controls (P = 4.7 × 10 -35 and P = 1.1 × 10 -24 , respectively). The initial DReSS feature was typically reported 2 to 8 weeks after initiating IL-1/IL-6 inhibition. In drug-reaction cases stopping versus not stopping IL-1/IL-6-inhibitor treatment, reaction-related features were indistinguishable, including pulmonary complication rates (75% [39 of 52] vs 76% [28 of 37]). Those stopping subsequently required fewer medications for treatment of systemic inflammation, had decreased rates of macrophage activation syndrome, and improved survival (P = .005, multivariate regression). Resolution of pulmonary complications occurred in 67% (26 of 39) of drug-reaction cases who stopped and in none who continued inhibitors., Conclusions: In systemic inflammatory illnesses, recognition of IL-1/IL-6-inhibitor-associated reactions followed by avoidance of IL-1/IL-6 inhibitors significantly improved outcomes., (Copyright © 2024 American Academy of Allergy, Asthma & Immunology. All rights reserved.)

Background: Although beta-lactams are 1 of the major causative agents of severe cutaneous adverse reactions (SCAR), their epidemiology and clinical aspects have been poorly studied. This study aimed to investigate the characteristics of SCAR caused by beta-lactams in the Korean SCAR registry. Methods: We retrospectively analyzed beta-lactam-induced SCAR cases collected from 28 tertiary university hospitals in Korea between 2010 and 2015. The SCAR phenotypes included Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), SJS-TEN overlap, and drug reaction with eosinophilia and systemic symptoms (DRESS). Beta-lactams were classified according to their chemical structures: penicillins, cephalosporins, and carbapenems. The causative beta-lactams, clinical and laboratory features, treatments, and outcomes were evaluated. Results: Among the 275 antibiotic-induced SCAR cases, 170 patients developed SCAR induced by beta-lactams. Beta-lactam antibiotic-induced SCAR showed more frequent SJS/TEN compared to SCAR induced by non-beta-lactam antibiotics (SJS/TEN/SJS-TEN overlap/DRESS: 36.5/11.2/5.9/46.5% vs. 23.8/10.5/2.9/62.9%, P = 0.049). Cephalosporin was the most common culprit drug. Particularly, 91 and 79 patients presented with SJS/TEN and DRESS, respectively. The odds ratio (OR) for poor prognosis, such as sequelae and death, was significantly increased in subjects with SJS-TEN overlap and TEN and carbapenem as culprit drug in the multivariate analysis (OR, 35.61; P = 0.016, OR, 28.07; P = 0.006, OR 30.46; P = 0.027). Conclusion: Among antibiotic-induced SCAR, clinical features were different depending on whether the culprit drug was a beta-lactam antibiotic or SCAR type. The poor prognosis was related to SJS-TEN overlap, TEN type, and carbapenem as the culprit drug.

Background: HLA‐B*58:01 is a well‐known risk factor for allopurinol‐induced severe cutaneous adverse reactions (SCARs). However, only a minority of HLA‐B*58:01 carriers suffer SCARs after taking allopurinol. The aim of this study was to investigate subsidiary genetic markers that could identify those at further increased risk of developing allopurinol‐induced drug reaction with eosinophilia and systemic symptoms (DRESS) in subjects with HLA‐B*58:01. Methods: Subjects with B*58:01 were enrolled (21 allopurinol‐induced DRESS and 52 allopurinol‐tolerant control). HLA‐A, ‐B, ‐C and ‐DRB1 alleles were compared. Comparison of risk between HLAs and allopurinol‐induced SCAR in separate populations was performed to support the results. Kruskal‐Wallis test, Pearson's chi‐square test, Fisher's exact test and binary logistic regression were used to analyze the risk of SCAR development. Results: Frequencies of A*24:02 (71.4 vs. 17.3%, p < 0.001, odds ratio [OR] = 12.0; 95% confidence interval [CI], 3.6–39.2) were significantly higher in B*58:01 (+) DRESS than B*58:01 (+) tolerant controls. In addition, DRB1*13:02 further increased the risk of DRESS. The phenotype frequency of A*24:02/DRB1*13:02 was significantly higher in the B*58:01 (+) DRESS group than in the B*58:01 (+) tolerant controls (52.4% vs. 5.8%, p < 0.001, OR, 66.0; 95% CI, 6.1–716.2). In 2782 allopurinol user cohort, the overall prevalence of DRESS was 0.22%, which increased to 1.62% and 2.86% in the presence of B*58:01 and B*58:01/A*24:02, respectively. Conclusion: The additional secondary screening with A*24:02 and DRB1*13:02 alleles may identify those at further increased risk of allopurinol‐induced DRESS in B*58:01 carriers. [ABSTRACT FROM AUTHOR]

Drug induced linear IgA bullous dermatosis (LABD) is a rare blistering disease that has been shown to be associated with the use of various medications. Although rarely seen together, some of the medications associated with LABD can lead to the syndrome drug reaction with eosinophilia and systemic symptoms (DRESS), which presents with fever, cutaneous eruption, and multi-organ involvement. We present a patient who developed fever and a generalized vesiculobullous eruption after recently starting amlodipine and meloxicam. Initial laboratory tests demonstrated elevated liver function tests, leukocystosis, and eosinophilia. Histopathologic examination of the punch biopsy revealed a bulla with sub-epidermal split and numerous neutrophils. Direct immunofluorescence demonstrated broad deposition of IgA along the dermal-epidermal junction. These findings were consistent with an overlap between LABD and DRESS. Drug induced LABD and DRESS are independently both rare diseases. It is even more uncommon to see the two concurrently in the same patient. In this patient, these two conditions were thought to be triggered by either amlodipine or meloxicam. Given the high mortality rate associated with DRESS, it is important to recognize the presentation and initiate the appropriate treatment plan as soon as possible.

BACKGROUND: Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a rare, severe adverse event during treatment with raltegravir. The occurrence of DRESS syndrome during treatment with other drugs is strongly associated with particular HLA alleles. METHODS: We performed HLA testing in 3 of the 5 patients previously reported to have developed raltegravir-induced DRESS syndrome and in 1 previously unreported patient. We then used virtual modeling to visualize interactions between raltegravir and the imputed HLA molecule. RESULTS: Five of the 6 patients who developed raltegravir-induced DRESS syndrome were African, and 1 was Hispanic. HLA typing was performed in 4 patients, all of whom carried both the HLA-B*53 allele and the HLA-C*04 allele to which it is commonly haplotypic. No other HLA alleles were shared by all of the tested patients. Given the approximate prevalence of HLA-B*53 carriage in African (20%) and Hispanic (6%) populations, the probability of all 4 patients being HLA-B*53 carriers, and 2 of 3 African patients being homozygous for HLA-B*53:01, is approximately 0.00002. CONCLUSIONS: These data implicate the prevalent African allele HLA-B*53:01 in the immunopathogenesis of raltegravir-induced DRESS syndrome. Although the immunopathogenic mechanisms are currently unknown, virtual modeling suggests that raltegravir may bind within the antigen binding cleft of the HLA-B*53:01 molecule, but not within the closely related HLA-B*35:01 molecule. Further studies are necessary to confirm the strength of the association between carriage of the HLA-B*53:01 allele and raltegravir-induced DRESS syndrome, and the potential utility of HLA screening before raltegravir treatment.

Abstract This study reports a patient with DRESS syndrome, associated with liver failure, treated with orthotopic liver transplantation.

Skin eruptions are among the most common drug adverse reactions; therefore, it is usual to face them in medical practice. A systematic approach based on early identification of the possible etiologic agent, the chronology of the exposure and the clinical characteristics of the reaction, allows the clinician to categorize the severity of the disease. With a high index of suspicion, it is possible to choose the appropriate laboratory tests, to optimize medical care and take the pertinent therapeutic and preventive measures for each case. The following narrative review is intended to cover the key aspects of the most serious drug eruptions and propose a simple approach for these in the emergency department. [ABSTRACT FROM AUTHOR]

Introduction. Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome (sy) to carbamazepine has a heterogeneous clinical presentation. The aim of this report is to indicate the efficacy of immunoglobulin G in the treatment of corticosteroidresistant DRESS sy. Case outline. An adolescent suffering from epilepsy treated with carbamazepine and Na-valproate was hospitalized for fever, elevated transaminases, lymphadenopathy, splenomegaly. There was an eruption of skin efflorescence daily. On the sixth day of hospitalization, the number of eosinophils increased to 24% (780/ml absolute number). There was no desired response to methylprednisolone during the first eight days of treatment or to prednisolone during further treatment, with concomitant administration of antihistamines from day one of hospitalization, to Na-valproate, metformin hydrochloride, elimination diets, and carbamazepine withdrawal. Significant clinical, hematologic, and biochemical improvement occurred the day after the first dose of intravenous immunoglobulin G (IVIG). Conclusion. We point out the need to change the DRESS sy treatment recommendations in favor of the IVIG (as soon as the third or fourth day of treatment) in patients in whom the treatment with corticosteroids has no effect. Until new cases of the proven role of IVIG in the treatment of DRESS sy are published, corticosteroids remain the first therapeutic choice.

IntroductionDrug reaction with eosinophilia and systemic symptoms syndrome is a potentially life-threatening cutaneous hypersensitivity reaction characterized by extensive mucocutaneous eruption, fever, hematologic abnormalities including eosinophilia and/or atypical lymphocytosis, and extensive organ involvement. The drugs most often responsible for causing drug reaction with eosinophilia and systemic symptoms syndrome are anticonvulsants, antimicrobial agents and antipyretic or anti-inflammatory analgesics. Although azithromycin is widely prescribed in clinical practice, serious cutaneous reactions from this agent have been rarely described. We report the first adult case of drug reaction with eosinophilia and systemic symptoms syndrome associated with azithromycin.Case presentationA 44-year-old previously healthy Caucasian man with history of tobacco use presented to his primary care physician with fever and productive cough. He was prescribed azithromycin, promethazine hydrochloride and dextromethorphan hydrobromide syrup. One week later, he developed a blistering erythematous rash over both hands, which over the next two weeks spread to involve nearly his entire body surface, sparing only his face. He was admitted to an outside hospital with signs of systemic inflammatory response syndrome and severe sepsis, presumably from a skin infection. Despite aggressive therapy he deteriorated, with worsening diffuse erythema, and was transferred to our institution. He developed multiple organ failure requiring ventilatory and hemodynamic support. Pertinent laboratory studies included a leukocytosis with a white blood cell count of 17.6 × 10(9)/L and 47% eosinophils. A skin biopsy showed evidence of spongiotic lichenoid dermatitis with eosinophils and neutrophils, compatible with a systemic drug-induced hypersensitivity reaction. Our patient was started on high-dose steroids and showed dramatic improvement within 48 hours.ConclusionsWe report the first adult case of drug reaction with eosinophilia and systemic symptoms syndrome associated with azithromycin exposure. Clinicians should be aware of this potentially devastating complication from this commonly prescribed medication.